Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma, characterized by high clinical and biological heterogeneity. Patients typically present with progressive lymphadenopathy, extranodal disease and may also experience fever, night sweats and unexplained weight loss. We report here the case of a 16-year-old female with osteoarticular pain, dizziness, and dyspnea on exertion. Clinical examination showed no lymphadenopathy. Complete blood count (CBC) revealed pancytopenia and marrow smears found to be hypocellular. Initial diagnosis favored secondary myelofibrosis. Diagnosis of bone marrow involvement by DLBCL was retained on bone marrow histology and immunohistochemistry which showed infiltration of large B lymphoid cells. The patient was treated by immunochemotherapy R-CHOP regimen. This case highlights a very rare and atypical circumstance of discovery of DLBCL with myelofibrosis as an initial symptom. Prognosis value of this presentation and management difficulties are also discussed.
References
[1]
Sehn, L.H. and Salles, G. (2021) Diffuse Large B-Cell Lymphoma. The New England Journal of Medicine, 384, 842-858. https://doi.org/10.1056/NEJMra2027612
[2]
Fall, S., Ndiaye, F.S.D., Niang, E.H.D., Sarr, K., Sène, A., et al. (2021) Profile of Adult Lymphomas in Sub-Saharan Africa: A Monocentric Study of Histoimmunochemical Aspects. Journal of Hematology and Blood Disorders, 7, 104.
[3]
Yao, Z., Deng, L., Xu-Monette, Z.Y., Manyam, G.C., Jain, P., Tzankov, A., Visco, C., Bhagat, G., Wang, J., Dybkaer, K., Tam, W., Hsi, E.D., van Krieken, J.H., Ponzoni, M., Ferreri, A., Møller, M.B., Winter, J.N., Piris, M.A., Fayad, L., Liu, Y., et al. (2018) Concordant Bone Marrow Involvement of Diffuse Large B-Cell Lymphoma Represents a Distinct Clinical and Biological Entity in the Era of Immunotherapy. Leukemia, 32, 353-363. https://doi.org/10.1038/leu.2017.222
[4]
Naithani, R., Tyagi, S. and Choudhry, V.P. (2008) Secondary Myelofibrosis in Children. Journal of Pediatric Hematology/Oncology, 30, 196-198. https://doi.org/10.1097/MPH.0b013e318161a9b8
[5]
Jiang, Y.-F., He, R.-Q., Li, Z.-Q., Gu, Y.-Y., Chen, G. and Ma, J. (2017) Diffuse Large B-Cell Lymphoma with Initial Symptoms of Myelofibrosis Treated with Rituximab: A Case Report and Literature Review. International Journal of Clinical and Experimental Pathology, 10, 7093-7099.
[6]
Riccardi, A., Ucci, G., Coci, A. and Ascari, E. (1988) Bone Marrow Fibrosis in Multiple Myeloma. American Journal of Clinical Pathology, 90, 753-754. https://doi.org/10.1093/ajcp/90.6.753a
[7]
Okabe, S., Miyazawa, K., Iguchi, T., Sumi, M., Takaku, T., Ito, Y., Kimura, Y., Serizawa, H., Mukai, K. and Ohyashiki, K. (2005) Peripheral T-Cell Lymphoma Together with Myelofibrosis with Elevated Plasma Transforming Growth Factor-Beta1. Leukemia & lymphoma, 46, 599-602. https://doi.org/10.1080/10428190400029809
[8]
Hagihara, M., Watanabe, S., Suyama, T. and Sato, K. (2005) Rinsho ketsueki. The Japanese Journal of Clinical Hematology, 46, 1129-1135.
[9]
Matsunaga, T., Takemoto, N., Miyajima, N., Okuda, T., Nagashima, H., Sato, T., Terui, T., Sasaki, H., Ohmi, N., Hirayama, Y., Tamura, Y. and Niitsu, Y. (2004) Splenic Marginal Zone Lymphoma Presenting as Myelofibrosis Associated with Bone Marrow involvement of Lymphoma Cells which Secrete a Large Amount of TGF-Beta. Annals of Hematology, 83, 322-325. https://doi.org/10.1007/s00277-003-0806-y
[10]
Etienne, A., Gruson, B., Chatelain, D., Garidi, R., Royer, B., Sevestre, H., Marolleau, J.P. and Damaj, G. (2009) Myelofibrosis-Associated Lymphoproliferative Disease: Retrospective Study of 16 Cases and Literature Review. Advances in Hematology, 2009, Article ID: 179847. https://doi.org/10.1155/2009/179847
[11]
Mondello, P. and Mian, M. (2019) Frontline Treatment of Diffuse Large B-Cell Lymphoma: Beyond R-CHOP. Hematological Oncology, 37, 333-344. https://doi.org/10.1002/hon.2613
